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However, the clinicopathologic aspects of PTCL-F are poorly described and the normal cell counterpart of this subgroup of lymphoma is still unknown.

Three patients with sequential biopsies disclosed clinical and histopathologic features of angioimmunoblastic T-cell lymphoma at initial presentation.

Our results show that this rare form of PTCL-F (1) has an immunophenotype indicative of derivation from TFH cells, (2) is associated with t(5;9) in a proportion of cases, and (3) shows some overlapping features with angioimmunoblastic T-cell lymphoma, raising the question of a possible relationship.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Angioimmunoblastic T-cell lymphoma is characterized by a paracortical proliferation of medium to large neoplastic T cells, often with clear cytoplasm, in a background of arborizing high endothelial venules, many surrounded by follicular dendritic cells (FDCs).

The authors stained a collection of 45 angioimmunoblastic T-cell lymphomas with CD21, CD23, CNA.42, cystatin A, and fascin for direct comparison of FDC staining characteristics in this setting.

CD21 highlighted the expected dendritic network of cell processes, within residual follicles and outside of follicles, often adjacent to proliferating vessels.

Cystatin A stained the cytoplasm of follicular dendritic cells within and outside of follicles; however, staining was often not sharply localized to dendritic cell processes, and scoring was further complicated by reactivity with other cell types in over half of the cases.

Likewise, fascin stained a variety of cell types, including strong staining of interdigitating dendritic-like cells, moderate staining of endothelial cells, and only weak staining of follicular dendritic cells within and outside of follicles.

[Other-IDs] NLM/ PMC2817630

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title]Angioimmunoblastic T-cell lymphoma with autoimmune thrombocytopenia: a report of two cases.

We report two patients, a 68-year-old man (Case 1) and a 66-year-old man (Case 2), with polyclonal gammopathy, lymphadenopathy, thrombocytopenia, and high platelet-associated IgG (PAIgG) level.

We initially diagnosed them as having angioimmunoblastic lymphadenopathywith dysproteinemia (AILD).

From confirmation of clear cells by careful observation and detection of rearrangement bands of T cell receptors by Southern blot hybridization analysis, we finally concluded that their diagnoses were compatible with angioimmunoblastic T-cell lymphoma (AILT).

AILT with autoimmune thrombocytopenia (AIT) is very rare, and all the reported cases were Japanese ones.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The germinal center T-helper cell has been proposed as the cell of origin for angioimmunoblastic T-cell lymphoma.

Our recent report of expression of CXCL13, a chemokine critical for germinal center formation and one of the most highly upregulated genes in the germinal center T-helper cell subset, in the majority of angioimmunoblastic T-cell lymphoma cases, provided further support for this theory.

By WHO classification criteria, 20 of the lymphoma cases were considered peripheral T-cell lymphoma, unspecified, and six were reclassified as angioimmunoblastic T-cell lymphoma after immunohistochemical detection of disorganized follicular dendritic cell meshworks.

The two peripheral T-cell lymphoma, unspecified cases that were positive for CXCL13 showed a Lennert lymphoma-like histology.

While these cases did not meet all histologic criteria for angioimmunoblastic T-cell lymphoma, they did show an increase in EBV-positive B cells, suggesting they may be histologic variants of angioimmunoblastic T-cell lymphoma.

In conclusion, CXCL13 expression is a distinctive feature of angioimmunoblastic T-cell lymphoma, providing further support for the germinal center T-helper cell as the cell of origin for this neoplasm.

Given its specificity when compared to cases of peripheral T-cell lymphoma, unspecified as well as paracortical lymphoid hyperplasia, it may be a useful marker in the diagnosis of angioimmunoblastic T-cell lymphoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Frequent occurrence of B-cell lymphomas in angioimmunoblastic T-cell lymphoma and proliferation of Epstein-Barr virus-infected cells in early cases.

Secondary lymphomas occurring in the setting of angioimmunoblastic T-cell lymphoma (AILT) are considered to be rare.

A previous study detected a dysregulated hypermutation process in B-cells of AILT.

The present study aimed at estimating the frequency of B-cell lymphomas in AILT.

By studying the expression of EBV and activation-induced cytidine deaminase (AID) as an indicator of hypermutating cells, we assessed whether B-cell lymphoproliferations in AILT were strictly associated with EBV and whether hypermutation might contribute to lymphomagenesis.

Among 161 cases of AILT, diagnosed between 1996 and 2005 at the lymph node registry, Frankfurt, Germany, 19 cases were detected that also had B-cell non-Hodgkin lymphoma (NHL) and two cases had classical Hodgkin lymphoma (HL).

AID was expressed in AILT in large cells disseminated in the tissue, implying that the process of somatic hypermutation is ongoing in AILT, although the GC architecture is disrupted.

In this study, we examined T-cell lymphomas for c-Maf and cyclin expression immunohistochemically.

Of 93 cases of T-cell lymphomas we investigated in the current study, c-Maf expression was seen in 23 out of 31 cases of AILT, 3 out of 11 of adult T-cell leukemia/lymphoma, 4 out of 19 of peripheral T-cell lymphoma, unspecified [PTCL(U)], and 0 out of 11 cases of mycosis fungoides, 0 out of 11 of anaplastic large cell lymphoma, and 1 out of 10 of extranodal NK/T-cell lymphoma, nasal type.

Double immunostaining in AILT revealed that the majority of c-Maf-positive cells were also positive for CD43 (MT1), CD45RO (UCHL-1), and CD4 but were negative for CD20 (L26).

Additionally, cyclins D1 and D2, which stimulate cell cycle progression, were overexpressed in a large number of the c-Maf-positive AILT samples.

These findings strongly suggest that CD4-positive neoplastic T cells in AILT show c-Maf expression and provide new insight into the pathogenesis of AILT suggesting c-Maf to be a useful diagnostic marker for AILT.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Patients with angioimmunoblastic T-cell lymphoma can have profound immune dysfunction and immunodeficiency.

Epstein-Barr virus-driven B-cell lymphoid proliferation can occur in angioimmunoblastic T-cell lymphoma, as in other immunodeficiency states.

However, few cases of Epstein-Barr virus-positive B-cell lymphoma arising in patients with preexisting angioimmunoblastic T-cell lymphoma have been reported.

We report a case of angioimmunoblastic T-cell lymphoma in which diffuse large B-cell lymphoma developed 56 months after the diagnosis of angioimmunoblastic T-cell lymphoma.

The patient survived for 9 years after the initial diagnosis of angioimmunoblastic T-cell lymphoma, and molecular studies performed on multiple biopsy specimens during this period revealed the dynamic nature of clonal lymphoid expansion.

Epstein-Barr virus latent membrane protein 1 and Epstein-Barr virus-encoded RNA were detected in the diffuse large B-cell lymphoma, suggesting that Epstein-Barr virus may have played a role in the pathogenesis of the diffuse large B-cell lymphoma.

RESULTS: Amongst the 29 cases of AITL studied, 75.9% (22/29) showed aberrant expression of CD10, while all except one of the controlled cases were negative, 82.8% (24/29) of the AITL cases expressed CXCL13, while all cases of peripheral T-cell lymphoma, unspecified were negative.

As for bcl-6 staining, although the highest percentage of bcl-6-positive cells was observed in AITL, the expression pattern was not useful in differentiating AITL from peripheral T-cell lymphoma, unspecified and lymphoid reaction.

Two of the cases, which contained obvious germinal centers, had the follicular dendritic cell meshwork extending beyond the lymphoid follicles.

[Title] A multivariate analysis for the survival of nodal peripheral T-cell lymphoma (PTCL).

However, the superiority of high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) has remained inconclusive.

We conducted a retrospective study of fifty six patients with three major nodal PTCL, histologically classified as PTCL-not specified (NOS, n=29), angioimmunoblastic T-cell lymphoma (AITL, n=19), and ALK-negative anaplastic large cell lymphoma (ALCL, n=8) who underwent ASCT (n=14) or not (n=42) after CT in Tokai University Hospital, Ebina General Hospital, and Hadano Red Cross Hospital, Kanagawa, Japan between 1997 and 2008.

High-dose chemotherapy followed by autologous haematopoietic stem cell transplantation, has been explored in recent years with little experiences.

The patients were stratified according to their International Prognostic Index (IPI), disease status at the time of transplant and histology type.

RESULTS: Twenty-nine subjects were identified, with a median age of 51; 13 patients had Anaplastic T cell, 18 patient had PTCL-nos, and 6 patients with angioimmunoblasticT cell lymphoma.

Seventeen patients (58.62%) presented with an aa IPI score greater than 2.4 patients were in complete remission, 15 at first relapse, 4 in greater than 1 episode, and 6 with refractory disease at the time of transplantion.Kaplan Meier overall survival (OS) 72 and relapse free survival (RFS) was 62 at 1 year respectively.

A multivariate analysis and new risk stratification based on the IPI score system and disease status at time of transplant were employed.

CONCLUSIONS: The status at time of transplant with new methods for evaluation of minimal residual disease may help in assessing outcome.

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(PMID = 27961010.001).

[ISSN] 1527-7755

[Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

We retrospectively analyzed the clinical course and prognosis of 11 patients with angioimmunoblastic T-cell Lymphoma (AILT).

The disease can be classified into three categories based on histological findings: 3 cases of AILT with hyperplastic germinal centers, 4 cases of typical AILT, and 4 cases of AILT with numerous clear cells.

As the initial therapy, 10 patients received combination chemotherapy and only 1 patient received autologous peripheral blood stem cell transplantation.

Patients with AILT demonstrating hyperplastic germinal centers and no bone marrow infiltration were able to achieve long-term survival.

In order to investigate the clinical features, pathological manifestation of this lymphoma, and to explore its therapy protocol, a 37-years old patient with AILT was investigated.

In conclusion, the AITL patient complicated with AIHA and PRCA was successfully diagnosed, the lymphonode biopsy and bone marrow smear showed more significant, the chemotherapy protocol of CHOP-E can give some effect to cure such angioimmunoblasticT cell lymphoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Bone marrow (BM) biopsy is often performed early in the evaluation of patients with angioimmunoblastic T-cell lymphoma (AITL), and may be the first diagnostic tissue sample; yet the BM histopathology associated with this disease has not been well described.

Seven (54%) were involved by AITL, which was characterised by paratrabecular and interstitial polymorphous infiltrates containing cytologically atypical lymphocytes, histiocytes and eosinophils.

As in lymph nodes, the lymphomatous infiltrate in some BMs contained numerous small or scattered large B cells, resembling either benign lymphoid aggregates or T cell rich large B cell lymphoma, respectively.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] In angioimmunoblastic T-cell lymphoma, neoplastic T cells may be a minor cell population. A molecular single-cell and immunohistochemical study.

The significance of T-cell proliferations in angioimmunoblasticlymphoma (AILD) is still enigmatic.

Although classified as a malignant T-cell lymphoma in the World Health Organisation lymphoma classification, some cases of AILD lack dominant T-cell clones.

In a previous study, based on single-cell polymerase chain reaction (PCR), we obtained similar results as studies of AILD using Southern blot or conventional PCR: some cases of AILD contained large T-cell clones, and, in other cases, T-cell clones were undetectable.

As in single-cell studies, only a limited number of cells could be investigated; thus, we wanted to gain more insight into the amount and distribution of tumour cells.

By applying triple immunofluorescent staining with antibodies directed against T-cell receptor Vbeta-family-specific epitopes, we investigated T-cell populations in AILD and their localisation in the tissue in relation to B cells (CD20) and follicular dendritic cells (CD21).

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

OBJECTIVE: To characterize angioimmunoblastic T-cell lymphoma (AILT) on morphological, immunohistochemical and molecular grounds and its association with Epstein-Barr virus (EBV) in Pakistani patients.

PATIENTS AND METHODS: Over a period of 11 years archival biopsy material of 13 AILT cases (lymph nodes), identified on the basis of histological and immunohistochemical criteria, using REAL and WHO classifications, were retrieved from the files of Department of Pathology.

Immunophenotyping was carried out by using CD45 (LCA), two T-cell markers CD45RO (UCHL1; monoclonal) and CD3 (polyclonal).

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Clinical features and treatment outcomes of angioimmunoblastic T-cell lymphoma.

The objective of this retrospective study was to investigate clinical features and treatment outcomes in patients with angioimmunoblastic T-cell lymphoma (AITL), data of which were collected over a 15-year period.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Diffuse large B-cell lymphoma in patient after treatment of angioimmunoblastic T-cell lymphoma.

Relatively few cases of Epstein-Barr (EBV)-positive B-cell lymphomas arising in patients with angioimmunoblastic T-cell lymphoma (AITL) have been reported.

We report a case of AITL in which diffuse large B-cell lymphoma arose 13 months after the initial diagnosis of AITL.

In a 36-year-old female patient, evaluated for moderate leukocytosis, peripheral and abdominal lymphadenopathy AITL was diagnosed in March 2008, based on results of fine-needle aspiration cytology (FNAC) of the enlarged cervical and supraclavicular lymph nodes.

The diagnosis was also confirmed by immunophenotyping and histopathology of the cervical lymph nodes.

CHOP-R chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab) was then administered, resulting in good partial response of the disease.

AITL is a rare lymphoproliferative disorder in which the neoplastic T-cells represent the minority of the lymph node cell population and almost all cases harbor EBV-infected B-cells.

Various authors postulated that immunodeficiency in AITL patients together with immunosuppressive effects of cytotoxic drugs, may be responsible for EBV-induced proliferation of latently or newly EBV-infected B-cells with eventual clonal selection and progression to aggressive B-cell lymphoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

In most cases of lymphomas with blood dissemination, the careful cytological analysis of peripheral blood smears provides a rapid orientation to diagnosis, even if the final subtyping is achieved by histology and eventually other techniques.

Here, we evaluated if the analysis of blood smears may suggest the blood dissemination of angioimmunoblastic T-cell lymphoma (AITL) and if CD10 expression on neoplastic T cells, as recently reported on AITL, may contribute to the diagnosis.

In all, 11 lymph nodes and six peripheral blood samples from 12 patients with AITL were studied using four-colour flow cytometry associated to histological, cytological and molecular data.

Interestingly, all blood smears showed atypical lymphoid cells and a fraction of T cells expressed CD10 with a mean percentage of 18.75% (range 5.00-47.00%), regardless of lymphocytosis level and of rate of CD10 T cells in corresponding lymph node.

In contrast, in all control samples (100), none CD10-positive T cell was identified.

Therefore, they ought to be explored in further studies when aggressive lymphoma, in particular with lymphopenia and circulating atypical cells, is suspected.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Reports focusing on the immunological microenvironment of peripheral T-cell lymphomas (PTCL) are rare.

Here we studied the reciprocal contribution of regulatory (Treg) and interleukin-17-producing (Th17) T-cells to the composition of the lymphoma-associated microenvironment of angioimmunoblastic T-cell lymphoma (AITL) and PTCL not otherwise specified on tissue microarrays from 30 PTCLs not otherwise specified and 37 AITLs.

We found that Th17 but not Treg cells were differently represented in the two lymphomas and correlated with the amount of mast cells (MCs) and granulocytes, which preferentially occurred in the cellular milieu of AITL cases.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Skin manifestations of angioimmunoblastic T-cell lymphoma (AITL) are frequent, sometimes as first manifestations of the disease.

In the absence of a specific marker for neoplastic cells, diagnosis of AITL in skin biopsies is often difficult.

A few CD10 lymphocytes were found in only 2 samples of the AITL group, the identification of which was hampered by the presence of a dermal CD10 cell population with dendritic features.

In another case, a diagnosis of cutaneous marginal zone B-cell lymphoma had been proposed.

In conclusion, this study shows that neoplastic AITL CXCL13 T cells localize in the skin and that accurate diagnosis of AITL lesions can be done in skin specimens using CXCL13 immunostaining on paraffin-embedded tissues.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title]Angioimmunoblastic T-cell lymphoma with hyperplastic germinal centres: a neoplasia with origin in the outer zone of the germinal centre? Clinicopathological and immunohistochemical study of 10 cases with follicular T-cell markers.

Angioimmunoblastic T-cell lymphoma is an aggressive peripheral T-cell lymphoma whose natural history is not fully understood.

The accurate recognition of Angioimmunoblastic T-cell lymphoma with pattern I remains a challenge and therefore the aim of this study is to phenotypically and morphologically characterize this variant with the use of the follicular helper T-cell (T(FH)) markers PD1, CXCL-13 and ICOS.

Molecular probe methods for the detection of T-cell and B-cell clonality, as well as in-situ hybridization probes for EBV RNA expression, were carried out to leave no question as to the establishment of the diagnosis in each case.

By contrast, CD10 was found to only weakly label the neoplastic T cells, with only 5-10% of the target cell population staining for this marker.

In conclusion we have shown that the T(FH) cells markers PD1, CXCL13 and ICOS are useful adjuncts in the diagnosis of Angioimmunoblastic T-cell lymphoma with hyperplastic germinal centres.

PD1 also highlighted the presence of neoplastic cells in the outer zone of lymphoid follicles, suggesting that Angioimmunoblastic T-cell lymphoma (pattern I) may originate from T(FH) cells in this region, in accordance with previous immunological studies.

As the majority of cases in our series presented clinically with advanced stage disease, progression from pattern I to classical Angioimmunoblastic T-cell lymphoma may represent histological evolution rather than clinical progression.

We conclude that the recognition and classification of angioimmunoblastic T-cell lymphoma in bone marrow are made difficult by the uncommon expression of CD10 (25%), rarity of follicular dendritic cells, and lack of CXCL13 expression at this site.

By contrast, programed death-1 immunohistochemical staining and double labeling using antibodies specific for BCL-6 and CD3 were helpful in appreciating the follicular T-helper cell immunophenotype of angioimmunoblastic T-cell lymphoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

BACKGROUND AND OBJECTIVES: In the present paper we report that SAP, an intracytoplasmic molecule that is involved in cell signaling, is an immunohistologic marker for germinal center T cells in paraffin-embedded tissue.

We document its expression, and also that of PD-1 (another recently described marker of germinal center T cells to which a new antibody has been raised), in normal and neoplastic lymphoid tissue to evaluate the suggestion that helper T cells within the germinal centers of human lymphoid tissue are the cell of origin of angioimmunoblastic T-cell lymphoma (AITL), and to assess the diagnostic value of these two markers.

DESIGN AND METHODS: Expression of SAP and PD-1 was investigated by immunohistochemistry in paraffin-embedded tissue sections and in cell lines.

Western blotting was performed on cell lines, and antibody specificity was confirmed by immunostaining of transfected cells.

RESULTS Screening on more than 500 lymphoma biopsies showed that 95% (40/42) of cases of AITL expressed at least one of these markers.

However, PD-1 and SAP were also found in a minority of cases of peripheral T-cell lymphoma other than AITL, in contrast to a report that the former marker is specific for AITL.

This observation raises the possibility that such non-angioimmunoblastic cases may be related to germinal center helper T cells.

They may also prove of value in the diagnosis of this disease since a negative reaction was rarely observed in this disorder.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

To evaluate the prognostic significance of clinicobiologic and pathological features in angioimmunoblastic T-cell lymphoma (AITL), 157 AITL patients were retrieved from the GELA LNH87-LNH93 randomized clinical trials.

[Title] Advances in the understanding and management of angioimmunoblastic T-cell lymphoma.

The recent identification of follicular helper T (T(FH)) cell as the cell of origin of this neoplasm represents a major step in our understanding of the pathobiological characteristics of the disease and should, in the future, clarify the diagnostic criteria for AITL and help to delineate its spectrum, especially from PTCL, not otherwise specified (PTCL, NOS).

Deciphering the pathogenesis of the disease is needed to identify targets for new therapies that are expected to improve the poor outcome of AITL patients, when treated with conventional chemotherapy regimens.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

To evaluate the clinical, pathological characters and prognosis of patients with angioimmunoblasticT cell lymphoma (AITL), the clinicopathologic features, immunophenotypes, therapy and survival rate of 12 AITL patients which were confirmed by pathologic examination were retrospectively studied.

The results indicated that main symptom was observed as general lymphadenopathy, however, 9 patients had fever.

The diagnosis of AITL was based on lymph-node biopsy.

The histopathologic characteristics of AITL showed the damage of normal lymphnode structure, the proliferation of immunoblastic cells and arborescent super vascularization.

All immunophenotypes were mature peripheral T-cellular.

In conclusion, most cases of AITL display an aggressive course, therefore, the disease progresses rapidly and has unfavorable prognosis, further studies are required to improve its therapy regimen.

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[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Clinical features and prognostic factors of angioimmunoblasticT cell lymphoma].

OBJECTIVE: To retrospectively analyze the clinical features and prognostic factors of patients with angioimmunoblastic T-cell lymphoma (AITL).

All of the patients received CHOP-like regimens as initial chemotherapy, including 4 once treated with radiotherapy and 1 with high dose therapy followed by autologous stem cell transplantation (HDT-ASCT) as upfront consolidation therapy.

B-cell, T-cell and NK-cell subgroup proportions in the peripheral blood were tested by flow cytometry in 6 patients.

With the median follow-up of 26 months, the overall 2-year survival and disease free survival (DFS) rates were 62.2% and 44.4%, respectively.

Unspecified and 10 angioimmunoblastic T-cell lymphomas were analyzed.

The most demonstrative histological features of both types of lymphomas were spectrum of small, medium and large lymphoid cells, lymphoid cells with irregular nuclei, presence of clusters of clear cells, arborizing endothelial venules, increased number of histiocytes, eosinophils and plasma cells.

METHODS: One hundred and fifteen cases of AITL, 30 cases of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) and 30 cases of reactive lymph nodes with paracortical hyperplasia (RH) encountered during the period from January, 1990 to January, 2008 were retrieved from the archival files of the Department of Pathology, West China Hospital of Sichuan University, China.

An increased risk of developing lymphoma has been indicated in Sjögren's syndrome (SS), and the lymphomas in SS are usually B-cell type in origin.

To the best of our knowledge, including our case, at least 11 out of 23 (48%) cases of T-cell lymphoma reported in association with SS, were AILD.

The fact that the development of B-cell lymphoma in SS is much more frequent than that of T-cell lymphoma, might be explained by differences in the situation between B and T cells, although the exact mechanism still remains uncertain.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

AngioimmunoblasticT cell lymphoma (AILT) is an aggressive T cell lymphoma with an incidence of approximately 1-2% of all non-Hodgkin lymphoma.

The detection of clonal T cell receptor (TCR) gene rearrangements helps in the diagnosis of T cell malignancies such as AILT, where morphological and immunohistological investigations are not always sufficient to reach a definitive diagnosis.

TCR beta (TCRB) and TCR gamma (TCRG) gene rearrangements were analysed from 17 WHO-defined cases of AILT by PCR for the presence of TCR clonality.

The results of this study emphasise that TCR clonality and oligoclonality is a diagnostic feature of AILT and that BV17S1 is over-represented with no other common molecular findings.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

We describe a 66-year-old man initially diagnosed with primary cutaneous marginal zone B-cell lymphoma who developed four additional monoclonal/monotypic B-cell lymphoid proliferations and a systemic angioimmunoblastic T-cell lymphoma over the course of 19 months.

Through retrospective analysis, we identified the evolution of a T-cell clone within the background of clinically and pathologically dominant cutaneous B-cell tumors.

In terms of clinical practice, this case supports that patients diagnosed with multiple clonal B-cell proliferation need thorough investigation and close clinical follow up to identify a coexistent or evolving systemic lymphoma, in particular, peripheral T-cell lymphomas of follicular T-helper cell type, such as angioimmunoblastic T-cell lymphoma.

Biologically, this case offers unique insight into the interactions between B-cell and T-cell lineages in lymphoid neoplasia.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] The frequency of B- and T-cell gene rearrangements and epstein-barr virus in T-cell lymphomas: a comparison between angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, unspecified with and without associated B-cell proliferations.

We report on a series of 58 cases of angioimmunoblastic T-cell lymphoma (AILT) and 59 cases of peripheral T-cell lymphoma, unspecified (PTCL-NOS).

Subsets of cases from both diagnostic groups were complicated by associated B-cell proliferations, and we performed B- and T-cell clonality studies and in situ hybridization for Epstein-Barr virus (EBV) to investigate the relationship between B-cell proliferation, B-cell clonality, and EBV.

Using multiplex polymerase chain reaction assays based on the BIOMED-2 collaborative study, we detected TCRgamma T-cell clones in 78 and 81% of AILT and PTCL-NOS cases, respectively, and IGH B-cell clones in 34 and 35% of AILT and PTCL-NOS cases, respectively.

The majority of cases contained EBV-positive cells, including 50% of AILT and 57% of PTCL-NOS cases, and cases with B-cell proliferations were more often EBV-positive.

Although a relatively high rate of B-cell clonality has been shown for AILT, our findings for PTCL-NOS differ from previous reports in that B-cell clonality was relatively frequent.

Overall, a positive B-cell clone correlated, in part, with the presence of a B-cell proliferation but not with EBV.

Our findings demonstrate that B-cell clonality is a common finding in AILT and PTCL-NOS, and its presence should not negate the diagnosis established by morphologic, immunophenotypic, and clinical findings.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] The Germinal centre-derived lymphomas seen through their cellular microenvironment.

The human lymph node is a complex tissue resulting from the microenvironmental organisation of different cell populations linked by topographical and/or functional relationships.

On the whole, GC exerts a role in B cell physiology and malignancy.

In GC-derived lymphomas, gene expression profiling studies have raised the possibility that survival of the affected patients may be associated with signatures preferentially expressed in non-malignant T cells and macrophages and/or dendritic cells.

Immunohistological analyses in lymphoma biopsy samples have confirmed that the biological behaviour and tumour progression may be influenced by the tumour microenvironment.

This review will examine GC-derived lymphomas, including follicular lymphomas, Hodgkin lymphomas and angioimmunoblastic T-cell lymphoma, through their integrated cellular microenvironment, highlighting those findings which may serve as a useful surrogate marker for tumour diagnosis or tumour progression, together with key molecules involved in tumour development.

We report an unusual case of angioimmunoblasticT cell lymphoma arising in the setting of 5 years of immunosuppressive treatment for progressive systemic sclerosis.

Phenotypical examination of the lymph node also revealed the predominance of CD4+ T cells in addition to the proliferation of follicular dendritic cells, but no light chain restriction in large B cell components.

Based on the combined data described here, our preferred diagnosis was angioimmunoblasticT cell lymphoma with Epstein-Barr virus-associated B cell lymphoproliferative disorder, the pathogenesis of which was suggested to be closely associated with immunosuppressive treatment for progressive systemic sclerosis.

Significant progress has been made in the understanding of AITL since its recognition as a clonal T-cell disorder with associated deregulation of B-cells and endothelial cells within a unique malignant microenvironment.

We discuss recent developments in the understanding of the pathogenesis of AITL at a cellular and molecular level, including the implication of the follicular helper T-cell as the corresponding cell of origin, the roles of Epstein-Barr virus, B-cell deregulation, angiogenesis, and other signaling pathways in AITL, and the therapeutic implications of these findings.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Clinicopathologic features and diagnosis of 18 patients with angioimmunoblastic T-cell lymphoma].

OBJECTIVE: To explore the clinical and pathologic features of angioimmunoblastic T-cell lymphoma(AITL) and provide evidence for diagnosis.

RESULTS: Characteristic features at the presentation of AITL included generalized lymphadenopathy, fever, splenomegaly, and skin rashes with polyclonal hyper-gammaglobulinemia and other hematological abnormalities (such as Coombs-positive hemolytic anemia), which often involved the bone marrow and had well-described histologic features.

CONCLUSION: Repeated lymphadenbiopsy is helpful for AITL diagnosis.

Routine histological and immunohistochemical examinations (especially including CXCL13) play significant role in the diagnosis and differential diagnosis of AITL.

Despite the presence of pleural effusion, the diagnosis of mesothelioma was initially established by cytologic ex-amination of pericardial fluid, due to the patient's critical cardiac condition requiring prompt subxiphoid pericardiocentesis.

CONCLUSION: CT as primary clinical manifestation of AILT is very rare.

This case reflects the differences in the underlying biology of AILT and consequently the vast spectrum of its clinical presentations.

Coexistence of AILT with malignant pleural mesothelioma is also extremely rare.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The immunohistochemical and pathologic findings from the biopsy specimens led to the diagnosis of angioimmunoblastic T-cell lymphoma (AILT) with a cluster of CD20-positive cells.

These findings indicated a diagnosis of angioimmunoblastic T-cell lymphoma complicated with EBV-associated B-cell lymphoma, and that immunodeficiency in AILT led to an expansion of EBV infected B-cells.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Allogeneic stem cell transplantation is able to induce long-term remissions in angioimmunoblastic T-cell lymphoma: a retrospective study from the lymphoma working party of the European group for blood and marrow transplantation.

Median age was 48 years (range, 23 to 68 years), 34 patients had received > or = two lines of chemotherapy before alloSCT, and 11 patients had experienced treatment failure with a prior autologous stem-cell transplantation.

Twenty-seven patients were allografted in chemotherapy-sensitive disease, and 18 were allografted in refractory disease.

RR was estimated as 16% and 20% at 2 and 3 years, respectively, and was lower in patients developing chronic graft-versus-host disease (cGVHD).

Both the lower RR after transplantation as well as the decreased RR in patients developing cGVHD after the alloSCT suggests the existence of a clinically relevant graft-versus-lymphoma effect.

These results suggest that the angiopoietin system may play an important role in the development of high vascularity in AILT lymph nodes.

Consequently, as neoplastic T-cells and follicular dendritic cells are both increased in AILT and may represent an important source of angiogenic mediators, targeting these cells with anti-angiogenic strategies might represent a novel therapy for AILT.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

AngioimmunoblasticT cell lymphoma is a distinct entity for which there is no standard therapy.

On the basis of the rationale that CsA may represent a novel drug for AITL, a disease with considerable immune dysregulation, and encouraging case reports, the authors have treated 12 patients with this agent.

Responding patients received a maintenance dose of 50 - 100 mg, with a gradual taper after a maximal response was achieved as tolerated.

By interrupting T-cell activation, CsA may alter the immune dysregulation that characterizes AILT.

The efficacy of CsA is being explored in patients with recurrent AILT in a prospective trial (ECOG 2402).

To characterize the structural anomalies of chromosomes associated with these disorders, we here determined chromosome copy number alterations (CNAs) and loss of heterozygosity (LOH) at >55,000 single nucleotide polymorphism loci for clinical specimens of AILT (n=40) or PTCL-u (n=33).

AILT- or PTCL-u-specific CNAs or LOH were also identified at 21 regions, some spanning only a few hundred base pairs.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Angioimmunoblasticlymphoma (AITL) is a nodal peripheral T-cell lymphoma characterized by a proliferation of arborizing vessels and hyperplastic follicular dendritic cells as well as a polymorphous lymphoid infiltrate including neoplastic cells with clear cytoplasm.

Adult T-cell leukemia/lymphoma (ATLL) is caused by the retrovirus human T-cell leukemia virus type I (HTLV-I), and the neoplastic cells are usually large and pleomorphic.

Recently, a rare morphologic variant of ATLL with AITL-like features has been reported.

Here, we presented a case of peripheral T-cell lymphoma with morphological features of AITL in Taiwan, a country non-endemic for HTLV, and the patient was seropositive for anti-HTLV antibody, which raised the possibility of ATLL with AITL-like features.

Our investigations indicated that in an HTLV-I non-endemic area, a peripheral T-cell lymphoma with typical morphologic and immunophenotypic features of AITL could be confidently diagnosed as AITL even if the patient was seropositive for anti-HTLV antibody.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Programmed death 1 is a marker of angioimmunoblastic T-cell lymphoma and B-cell small lymphocytic lymphoma/chronic lymphocytic leukemia.

PD-1 expression was recently reported in some T-cell non-Hodgkin lymphoma (NHL) subtypes, but the expression profile of PD-1 and its ligands (PD-L1 and PD-L2) in B-NHLs remains largely to be characterized.

A series of 161 lymphoma tissue and 11 blood samples was analyzed using either immunohistochemistry or flow cytometry.

In B-NHLs, PD-1 was mainly expressed in reactive T cells; but expression was also noted in neoplastic B cells from small lymphocytic lymphoma (SLL, 12/13), grade III follicular lymphoma (3/3), and diffuse large cell lymphoma (2/25).

Although many studies of tumor-associated macrophage (TAM) populations in various malignant tumors have been published, only a few have dealt with activation of macrophage phenotypes such as M1 and M2 in tumor tissue.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Angioimmunoblastic T-cell lymphoma (AILT) is an aggressive peripheral T-cell lymphoma that is frequently accompanied by a cutaneous eruption.

Here, we review the previously reported histologic patterns and present a case of AILT involving the skin with a unique histologic appearance of necrotizing granulomas with abundant histiocytes and eosinophils, mimicking an infectious etiology.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] The inducible T-cell co-stimulator molecule is expressed on subsets of T cells and is a new marker of lymphomas of T follicular helper cell-derivation.

BACKGROUND: T follicular helper (T(FH)) cells reside in the light zone of germinal centers and are considered the cell of origin of angioimmunoblastic T-cell lymphoma.

Recently, CXCL13, PD-1 and SAP were described as useful markers for T(FH) cells and angioimmunoblastic T-cell lymphoma but also reported in some peripheral T-cell lymphomas, not otherwise specified.

DESIGN AND METHODS: In the present study the expression pattern of ICOS protein was investigated by immunohistochemistry-based techniques in routine sections of normal lymphoid tissues and 633 human lymphomas.

In lymphomas, ICOS expression was confined to angioimmunoblastic T-cell lymphoma (85/86), peripheral T-cell lymphomas of follicular variant (18/18) and a proportion of peripheral T-cell lymphomas, not otherwise specified (24/56) that also expressed other T(FH)-associated molecules.

CONCLUSIONS: ICOS is a useful molecule for identifying T(FH) cells and its restricted expression to angioimmunoblastic T-cell lymphoma and a proportion of peripheral T-cell lymphomas, not otherwise specified (showing a T(FH)-like profile) suggests its inclusion in the antibody panel for diagnosing T(FH)-derived lymphomas.

Our findings provide further evidence that the histological spectrum of T(FH)-derived lymphomas is broader than previously assumed.

We studied 15 patients who developed an EBV-negative B-cell proliferation or malignant lymphoma associated with PTCL.

The B-cell component was intermingled with the PTCL in all patients and it was classified as clonal/monotypic plasma cell proliferation in 8 lesions, clonal/monotypic large B-cell proliferation in 4 patients, and B-cell lymphoma with plasmacytic/plasmablastic differentiation in 3 patients.

Two patients had 2 clonally unrelated plasma cell proliferations associated with the same PTCL.

Sequential samples in 7 patients showed persistence of the PTCL and the B-cell component in 4, the PTCL without the B-cell lymphoma in 2, and progression of the B-cell neoplasm in 1.

In conclusion, EBV-negative clonal or mononotypic B-cell proliferations in patients with PTCL present with a spectrum of lesions ranging from plasma cell proliferations to overt lymphomas with plasmacytic/plasmablastic features.

Certain subtypes, such as mantle cell, lymphocytic or follicular lymphoma, were found to express PKC beta(2) in > 90% of the samples.

Most angioimmunoblastic T-cell lymphomas, lymphoblastic T-cell lymphomas and marginal zone/mucosa-associated lymphoid tissue (MALT) lymphomas were labelled with anti-PKC betaII antibody, but the pattern of expression was more heterogeneous in these subtypes.

A minority of diffuse large B-cell lymphomas were stained and most plasma cell malignancies were negative.

None of the cases of Hodgkin's disease and anaplastic large cell lymphoma expressed PKC beta(2).

In this study, the authors analyzed the clinical behaviors of PTCLs and diffuse large B-cell lymphoma (DLBCL).

METHODS: The authors compared the characteristics and outcomes of 59 patients with PTCLs, including 33 angioimmunoblastic T-cell lymphomas and 26 unspecified peripheral T-cell lymphomas, with the characteristics and outcomes of 193 patients with DLBCLs who were treated in the era before rituximab.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Epithelioid cell granuloma occurs in association with many neoplasms including lymphoma.

However they have rarely obscured the microscopic features of a lymphoma.

We report on a case where a florid epithelioid cell granulomatous reaction caused difficulty in interpretation and delayed the final diagnosis of a case of peripheral T cell lymphoma of the angioimmunoblastictype.

In 2 patients who had failed to respond to conventional chemotherapies, the circulating lymphoma cells rapidly disappeared after the initiation of CyA, and one of these patients demonstrated a durable CR.

The remaining one patient with advanced AITL did not respond to CyA and died of disease progression.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

OBJECTIVES: Angioimmunoblastic T-cell lymphoma (AIL) is a rare lymphoma with a poor prognosis and no standard treatment.

Here, we report our experiences with 19 patients treated with high-dose chemotherapy and autologous stem cell transplantation (HDC/ASCT) within the GELTAMO co-operative group between 1992 and 2004.

Most patients received peripheral stem cells (90%) coupled with BEAM or BEAC as conditioning regimen (79%).

After a median follow-up of 25 months, eight patients died (seven of progressive disease and secondary neoplasia), while actuarial overall survival and progression-free survival at 3 yr was 60% and 55%.

CONCLUSIONS: More than half of the patients with AIL that display unfavourable prognostic factors at diagnosis or relapse would be expected to be alive and disease-free after 3 yr when treated with HDC/ASCT.

Patients who are transplanted in a refractory disease state do not benefit from this procedure.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

While recent evidence suggests that the Epstein-Barr virus and B cell disregulation are implicated in the disease's pathogenesis, their mechanistic roles remain largely unknown.

The prognosis with traditional chemotherapy has been poor, but improved understanding of the disease's pathobiology has led to several promising novel therapeutic strategies.

RECENT FINDINGS: The recent finding of overexpression of the chemokine CXCL13 by the neoplastic cells of angioimmunoblasticT cell lymphoma suggests that it is derived from follicular helper T cells.

Novel therapeutic strategies including immunomodulation with agents like cyclosporine and angiogenesis inhibition with drugs such as bevacizumab are being investigated, and show early promise in this disease.

SUMMARY: Diseases such as angioimmunoblasticT cell lymphoma can help illuminate the biology of the normal immune system.

Significant progress has been made in understanding the biology of angioimmunoblasticT cell lymphoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Therapy of peripheral T/NK neoplasms.

These diseases have a geographic variation, with more nodal disease in North America and Europe, including peripheral T cell lymphomas, unspecified, anaplastic large cell lymphoma, and angioimmunoblasticT cell lymphoma; and more extranodal disease in Asia due to Epstein-Barr virus-related nasal NK/T lymphoma and human T-cell leukemia virus (HTLV)-1-associated adult T cell leukemia/lymphoma.

The prognosis in most peripheral T/NK neoplasms is poor, with 5-year survival less than 30%.

Progress has been slow due to the rarity of the diseases, geographic variation, relative chemoresistance, and lack of randomized trials.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The association of Epstein-Barr virus (EBV) with human immunodeficiency virus-negative T-cell lymphoma was examined in 68 patients using the polymerase chain reaction (PCR) with DNA obtained from formalin-fixed paraffin-embedded tissues and an in situ hybridization technique.

The 5-yr survival rate was 28% for peripheral T-cell lymphomas overall, 0% for NK/T-cell lymphomas, 38% for AILTs and 28% for other types of peripheral T-cell lymphoma.

The difference in the overall survival rate between NK/T-cell lymphoma and non-NK/T-cell lymphoma was significant (P = 0.0498 by Log-rank test).

Among peripheral T-cell lymphoma patients overall, the group severely infected with EBV (EBER-ISH ++) had a lower 5-yr survival rate (8%) than the group slightly (EBER-ISH +) or not infected (38%; P = 0.0013).